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CME ARTICLE |
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Advances in diaper technology |
p. 83 |
Sahana M Srinivas, Sandipan Dhar DOI:10.4103/2319-7250.179412 Diapering practices vary among different countries from the use of cloth diapers to the modern disposable diaper. In the last few decades, diaper technology has advanced significantly thus decreasing the prevalence of diaper dermatitis. Recent innovative techniques used in the manufacturing of diapers include incorporation of superabsorbent polymer gel that can absorb 30 times its weight in liquid. Recently, smart diapers have been developed which not only prevents diaper dermatitis but significantly has reduced the burden on parents. This article reviews how changes in disposable diaper technology have improved diapering practices. |
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REVIEW ARTICLE |
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Acitretin in pediatric dermatoses  |
p. 87 |
Manjyot Gautam, Harsh Tahiliani, Nitin Nadkarni, Sharmila Patil, Kiran Godse DOI:10.4103/2319-7250.179415 Acitretin, a synthetic retinoid and the active metabolite of etretinate has been increasingly used over the past two decades. It has proved effective in the treatment of many conditions associated with hyperkeratosis and dyskeratosis. A Google scholar search for the use of acitretin in pediatric dermatoses was done using the words “pediatric dermatoses,” “acitretin,” “etretinate,” “systemic retinoids,” “psoriasis,” “pityriasis rubra pilaris,” “ichthyoses,” “disorders of keratinization,” “Darier's disease,” “palmoplantar keratoderma,” “verrucae,” “lichen planus,” “lupus erythematosus,” and “lichen sclerosus.” All the articles were retrieved and classified into review articles, studies, double-blinded trials, and case reports. The final data were then analyzed and presented in a narrative fashion. It has been found that acitretin is useful in a number of pediatric dermatoses. It is preferred over other drugs in pustular psoriasis. Good results can be obtained in various disorders of keratinization, and it may even prove life-saving in conditions like harlequin ichthyosis. However, long-term maintenance therapy is required and exacerbations are known on discontinuing the drug. It can also be used as alternative therapy for many other pediatric dermatoses where the primary treatment has failed. Acitretin should be used even in children for the proper indications. However, proper clinical and laboratory surveillance has to be maintained in patients on long-term acitretin. |
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ORIGINAL ARTICLES |
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Clinico-mycological study of onychomycosis in children from a tertiary care center |
p. 95 |
Shazia Jeelani, Audil Mohmad Lanker, Nasir Jeelani, Qazi Masood Ahmed, Tawhida Fazili, Humera Bashir DOI:10.4103/2319-7250.179411 Introduction: Onychomycosis is rare in children as compared to adults and hence the presence of any nail changes in children needs careful examination and carrying out proper diagnostic tests to cure this treatable cause of dystrophic nails.
Aim: The study was undertaken to study in detail the clinico-mycological aspects of onychomycosis in children.
Materials and Methods: A
prospective hospital-based cross-sectional study was done on ninety children with a strong clinical suspicion of onychomycosis during a period of 2 years. All the children were subjected to three diagnostic tests, namely direct microscopy, fungal culture, and histopathological examination using periodic acid Schiff staining (HPE-PAS).
Results: Distal and lateral subungual onychomycosis is the most common type (82%), and toe nails (57.78%) are more commonly involved than fingernails. Direct microscopy yielded results in 62%, culture in 64%, and HPE-PAS in 71.1%. Dermatophytes were the most common group of organisms isolated (58%) with Trichophytonmentagrophytes as the most common isolate (31%). However, nondermatophytic molds and Candida species also accounted for 25% and 17% of the culture positive cases in children.
Conclusion: As children <18 years constitute one-third of our population, we suggest the need for careful evaluation of onychomycosis in this subgroup. |
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A clinico-epidemiological study on childhood vitiligo |
p. 101 |
Neerja Puri DOI:10.4103/2319-7250.179410 Introduction: Vitiligo is a disorder of melanocytes which may have a significant effect on the psyche of a patient.
Aims: To study the clinical profile of the patients with childhood vitiligo and see the association of vitiligo with other autoimmune diseases.
Methods: A randomized controlled prospective study was done of thirty patients of childhood vitiligo aged below14 years of age.
Results: Vitiligo vulgaris was the most common type of vitiligo seen in 50% children followed by focal and segmental vitiligo in 20% each followed by acrofacial vitiligo in 10% children. |
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Successful treatment of refractory childhood vesiculobullous disorders with rituximab: A study of five cases |
p. 104 |
Jeta Y Buch, Ranjan C Raval DOI:10.4103/2319-7250.175664 Introduction: Rituximab, a chimeric monoclonal anti-CD20 antibody, has shown efficacy as an adjuvant in the treatment with refractory vesiculobullous disorders. We, hereby, present a study of five pediatric patients of extensive vesiculobullous disorders showing resistance to conventional therapy of 40 mg of prednisolone daily and treated effectively with rituximab as an adjuvant.
Aim of the Study: To study the efficacy, safety, and clinical outcome of rituximab in refractory autoimmune vesiculobullous disorders.
Method: Five patients (3: Pemphigus vulgaris, 1: Pemphigus foliaceous, 1: Chronic bullous disease of childhood [CBDC]) were selected for treatment with rituximab after confirmation with tzanck, biopsy, direct immunofluorescence (DIF) and desmoglein (DSG) level. Three hundred milligram intravenous infusion in children over 4–5 h duration. Two doses were given at 15 days interval. DSG 1 and 3 and differential item functioning were repeated after 1-month of the second dose of rituximab. Follow-up (weekly for 1-month, fortnightly for next 2 months). Two patients (1: Pemphigus vulgaris, 1: CBDC) showed relapse after 6 months. So, they were given two more doses of rituximab at an interval of 15 days.
Observation: All five patients showed complete remission during the 6 months follow-up period, along with a consensual decline of the serum anti-DSG titers.
Conclusion: Rituximab can be considered as an effective adjuvant therapy when treating resistant cases of autoimmune blistering diseases in pediatric patients. However, more number of patients and long-term follow-up is required to draw a definite conclusion. |
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Phyrnoderma: Associated nutritional co-morbodities among children attending at Nutrition Clinic, Osmania General Hospital, Hyderabad, India |
p. 108 |
Amruth Rao Parveda, Brahmaiah Upputuri, R Hemalatha DOI:10.4103/2319-7250.175657 Introduction: Phrynoderma, meaning toad skin, is a form of follicular hyperkeratosis. Multiple nutritional deficiency disorders have been implicated in the etiology of phrynoderma. It is commonly seen in the children belonging to low socio-economic strata, clinically characterized by discrete, follicular, skin colored papules with keratotic plugs distributed over elbows, knees, extensor extremities and buttocks. The present study aims to determine the clinical presentation and associated nutritional co morbidities in diagnosed phrynoderma cases.
Materials and Methods: A series of 339 clinically diagnosed phyronderma cases attending the Nutrition Unit of tertiary care hospital during a period from September 1995 to March 2014 were retrospectively reviewed. All cases were reviewed for clinical history, cutaneous examination findings including associated signs of nutritional deficiencies.
Results: In a total of 339 patients of Phrynoderma studied, there were 195 males and 144 females. Majority of patients were in 6-10years age group. The lesions were distributed bilaterally and symmetrically in 196 (57.8%) patients. The lesions were localized predominantly to elbow followed by extremities in upper body and knees in lower body followed by buttocks. A total of 16.2% patients had associated ocular and skin findings and 13.3% of patients had the signs of Vitamin A deficiency.
Conclusion: Phyrnoderma is a disorder of young children and adolescents and can be considered as multifactorial disease involving deficiencies of various nutrients along with local factors such as pressure and friction responsible for pathogenesis of the disease. |
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CASE REPORTS |
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Congenital self-healing reticulohistiocytosis in a newborn: A rare variant |
p. 112 |
Shailvi Banka, Aparesh Patra, Sandip Gupta, SK Masud Hassan DOI:10.4103/2319-7250.179413 Congenital self-healing reticulohistiocytosis or congenital self-healing Langerhans cell histiocytosis (LCH) or Hashimoto-Pritzker syndrome is a rare, benign, auto involuting form of LCH. It usually presents at birth or soon thereafter with spontaneously regressing multiple or solitary dusky papules or nodules. The actual incidence remains unknown probably because of its benign and self-limiting nature. Herein, we present a similar case present since birth because of its rarity in nature. |
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Reiter's disease in a 13-year-old girl |
p. 114 |
Alia Abbas Rizvi, Amrinder J Kanwar, Shitij Goel, Seema Dua DOI:10.4103/2319-7250.175653 Reiter's disease or reactive arthritis is characterized by the classic triad of urethritis, arthritis, and conjunctivitis, usually following a genitourinary or gastrointestinal infection. Reiter's syndrome has been reported in children, but they usually do not manifest with the typical triad. Only a few such cases have been reported, and these have affected males predominantly. Furthermore, human leukocyte antigen B27 association in children is not well-established. However, in our case, it was positive. We, herein, report a case of a 13-year-old girl who presented with redness of eyes and joint pains followed by skin involvement is reported. She was managed with topical steroids and keratolytics along with oral nonsteroidal anti-inflammatory drugs and showed complete resolution in 3 weeks. |
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Scrofuloderma associated with lupus vulgaris and tuberculosis verrucosa cutis in an immunocompetent boy |
p. 117 |
Angoori Gnaneshwar Rao DOI:10.4103/2319-7250.175662 Cutaneous tuberculosis is protean and diverse in its clinical manifestations, it usually manifests as a single clinical morphological form. However, three different morphological forms of cutaneous tuberculosis such as scrofuloderma (SFD), lupus vulgaris (LV), and tuberculosis verrucosa cutis (TVC) may coexist in the same patient. Herein, we present coexistence of SFD, LV, and TVC in a 14-year-old boy, initially developed SFD in the left axilla and subsequently developed LV of the left elbow followed by TVC involving pubic region. Typical clinical morphology and epithelioid cell granuloma with Langhans giant cells on histopathological examination of the biopsy taken from the plaque of elbow and pubic region corroborated in establishing the diagnosis of SFD, LV, and TVC. The considerable clinical improvement was observed after 12 weeks of instituting four drugs antitubercular treatment. |
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Unilateral proteus syndrome in a neonate: A very rare presentation |
p. 121 |
Chinmay Kar, Shyamapada Singh, Syamal Sardar, Gauranga Biswas, Ramadas Murmu DOI:10.4103/2319-7250.175659 Proteus syndrome (PS) is a rare sporadic disorder with postnatal asymmetric overgrowth from any of the three germinal layers. The tissue overgrowth may present at birth but becomes more conspicuous with the progression of age. Though it apparently can affect any tissue, it commonly involves skin, bones, and central nervous system. The PS is difficult to diagnose as any part of the body is affected along with several complications. To overcome the diagnostic confusion, there are three general mandatory criteria with at least one of the three category signs designated as specific criteria according to First National Conference on PS. We diagnosed our case by connective tissue nevus in sole with three general criteria. Huge cerebriform nevus sebaceous as epidermal nevus was not found previously. A few neonatal PS cases were reported. All the lesions were unilaterally distributed. Our case was neonatal PS with unilateral distribution. We report this case because of its rarity. |
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Successful outcome of lamellar ichthyosis with oral retinoid therapy: A series of six cases  |
p. 125 |
K Pratyusha, P Divya Sree, BSN Reddy DOI:10.4103/2319-7250.172464 Lamellar ichthyosis (LI) is a rare skin disorder inherited as an autosomal recessive trait. Affected babies are born in a collodion membrane that sheds gradually revealing extensive scaling of the skin. The present communication is intended to provide the details of oral isotretinoin therapy in the management of LI in six children. The dose of oral isotretinoin which produced maximum clearing of the lesions with minimum side effects was found to vary from 0.6 mg/kg to 1 mg/kg body weight among these children and the duration of therapy lasted from 6 to 16 weeks. Therapeutic improvement varied from excellent in four and mild to satisfactory in two children and cheilitis of the lips was observed to be the main side effect. |
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A curious case of the necklace hair |
p. 129 |
Mukherjee Samipa Samir, BS Chandrashekar, R Pavan Raj, Chaithra Shenoy DOI:10.4103/2319-7250.175660 Monilethrix is a rare autosomal dominant inheritable hair shaft defect with variable penetrance. The diagnosis is confirmed by the characteristic beaded appearance of the hair under light microscopy which occurs due to defective keratinization. We hereby describe a case of monilethrix syndrome with keratotic follicular papules, moniliform hair, and koilonychias in an 8-year-old Indian female child. A rare association of trichorrhexis nodosa was also noted in our case. |
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A rare association of congenital melanocytic nevus and lissencephaly in childhood seizure |
p. 132 |
Sarita Kalwaniya, Pradeep Choudhary, Harsh Vardhan Khokhar, Devendra Yadav DOI:10.4103/2319-7250.175655 Dermatological and imaging evaluation of a 2-year-old male child with refractory seizures and developmental delay revealed an interesting and first of its kind association of congenital melanocytic nevus and classical lissencephaly. |
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Sweet's syndrome leading to acquired cutis laxa in a child (Marshall's syndrome): A rare case report |
p. 135 |
H Bangaru, KAK Surendran, BL Nanjundaswamy, B Vijaya DOI:10.4103/2319-7250.172467 Sweet's syndrome is very rare in children, fewer than 80 cases are reported in literature. The lesions usually resolve either spontaneously or after treatment without scarring. Marshall's syndrome is characterised by acute inflammatory skin lesions with subsequent development of localised elastolysis without any internal organ involvement. It is an extremely rare condition in children. There are very few cases reported as Sweet's syndrome leading to acquired cutis laxa - Marshall's syndrome. Sweet's syndrome shows excellent response to systemic steroids, whereas there is no satisfactory treatment for Cutis laxa. |
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Bullous systemic lupus erythematosus: An uncommon diagnosis in an 8-year-old girl |
p. 139 |
Uttam Lenka, Dipayan Sengupta, Souvik Sardar, Sarmishtha Ghosh DOI:10.4103/2319-7250.172468 Bullous systemic lupus erythematosus (BSLE) is a rare manifestation of SLE, characterized clinically by tense bullae involving photo-exposed areas and histologically by subepidermal bullae with predominantly neutrophilic infiltrate and immunoglobulin (IgG, IgA, and IgM) and C3 deposition at the dermo-epidermal junction indirect immunoflurosence. Specific diagnostic criteria for this subtype have been developed. However, the presentation in children is relatively uncommon. We report an 8-year-old girl diagnosed by histopathology and immunological examination as a case of BSLE without any clinical feature of SLE except photosensitivity. This highlights the importance of considering BSLE as a differential diagnosis in case of children presenting with tense bullae. |
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Loose anagen hair syndrome with diffuse woolly hair phenotype: A rare association |
p. 142 |
Arshdeep , Rahul Mahajan, Dipankar De, Sanjeev Handa DOI:10.4103/2319-7250.175656 Loose anagen hair syndrome (LAHS) is an underestimated cause of noncicatricial alopecia among children, manifesting as thin, sparse or fine hair. We report a case of LAHS clinically presenting as diffuse woolly hair, an association rarely described in the literature. In addition, we review the clinical as well as genetic link between these two enigmatic hair disorders and hypothesize that both may be associated in a yet unknown manner. |
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Erythrokeratodermia variabilis |
p. 145 |
Annasaheb Kadam, Swagata A Tambe, Dipti Das, Chitra S Nayak DOI:10.4103/2319-7250.172469 Erythrokeratodermia variabilis is a rare keratinization disorder characterized by two types of skin lesions, stationary hyperkeratotic scaly plaques, and migratory erythematous bizarre patches. Herein, we report a case of a 5-year-old female child who presented with pruritic well-defined, reddish brown hyperkeratotic plaques on trunk, axillae, neck, and extremities in bilaterally symmetrical distribution and had shown excellent improvement with isotretinoin. |
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A pediatric case of disseminated mutilating lupus vulgaris: A disgrace for society |
p. 148 |
Suresh Kumar Jain, Prafull Mehta, Savera Gupta, Ramesh Kumar DOI:10.4103/2319-7250.175661 Lupus vulgaris (LV) is a chronic and progressive form of cutaneous tuberculosis. The lesions may sometimes be associated with extensive destruction of tissue resulting in marked disfigurement and morbidity. A high index of suspicion is crucial for early diagnosis and treatment and thereby prevention of cosmetic deformity. Lupus vulgaris presenting as disseminated mutilating lesions in a child is uncommon, especially in today's era. Herein, we report an unusual case of lupus vulgaris with coexistence of multiple ulcerative mutilating lesions over face and classical plaque over distant site (right thigh) in a 9 year old girl. |
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Congenital varicella syndrome |
p. 151 |
Atul Chaudhary, Bela Padhiar, Umesh Karia, Kirti Parmar, Bela Shah DOI:10.4103/2319-7250.172472 A 7 month old female presented with localized scarring over right buttock and right leg with hypoplasia of right lower limb. Mother had history of chicken pox at tenth week of pregnancy. We confirm the diagnosis of varicella zoster syndrome clinically. |
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LETTERS TO EDITOR |
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Tinea corporis in an 8-day-old infant |
p. 153 |
Alia A Rizvi, Amrinder J Kanwar DOI:10.4103/2319-7250.175652 |
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Harlequin baby: A rare clinical entity |
p. 154 |
Shibani Pal, Shatanik Sarkar, Chaitali Patra, Angshumitra Bandyopadhyay DOI:10.4103/2319-7250.172475 |
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Juvenile systemic sclerosis in 11-year-old male child responding to methotrexate |
p. 156 |
Rohini P Gaikwad, Mukherjee Samipa Samir DOI:10.4103/2319-7250.175654 |
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Diagnostic dilemma between linear psoriasis and inflammatory linear verrucous epidermal nevus |
p. 159 |
Nibir Biswas, Dipayan Sengupta, Ishad Agarwal, Prerna Pathak DOI:10.4103/2319-7250.172476 |
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Extensive lupus vulgaris: A variant of extrapulmonary tuberculosis |
p. 161 |
Savita Arya, Ramesh Kumar Kushwaha, Moti Lal Bunkar, Suresh Jain DOI:10.4103/2319-7250.172477 |
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